Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literature

Fibrous dysplasia (FD) is a benign medullary fibro-osseous anomaly that compromises the mechanical strength of bones, especially the long bones that bear strong mechanical stresses. It can lead to an inability to remodel immature bone into mature lamellar bone, resulting in inappropriate bone alignm...

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Main Authors: Elio Paris, Giacomo De Marco, Oscar Vazquez, Sana Boudabbous, Christina Steiger, Romain Dayer, Dimitri Ceroni
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-01-01
Series:Frontiers in Pediatrics
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Online Access:https://www.frontiersin.org/articles/10.3389/fped.2024.1505766/full
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author Elio Paris
Giacomo De Marco
Oscar Vazquez
Sana Boudabbous
Christina Steiger
Romain Dayer
Dimitri Ceroni
author_facet Elio Paris
Giacomo De Marco
Oscar Vazquez
Sana Boudabbous
Christina Steiger
Romain Dayer
Dimitri Ceroni
author_sort Elio Paris
collection DOAJ
description Fibrous dysplasia (FD) is a benign medullary fibro-osseous anomaly that compromises the mechanical strength of bones, especially the long bones that bear strong mechanical stresses. It can lead to an inability to remodel immature bone into mature lamellar bone, resulting in inappropriate bone alignment in response to mechanical stresses. This case study describes a rare case of polyostotic FD presenting with an epiphyseal lesion of the proximal femoral head in its weight-bearing zone, accompanied by an unconventional femoral malrotation. The present case leads us to recommend that clinicians should not underestimate the occurrence of other deformities, such as the retrotorsion or flexion deformities that can compromise bone structure and the hip's biomechanics. Finally, the involvement of the epiphysis is probably more common than usually thought, introducing an additional complexity since juxta-articular lesions in weight-bearing joints may collapse, compromising articular congruence and function. To minimise this risk, bone scintigraphy and MRI should play a critical role in the patient's workup, evaluation, prognosis and follow-up.
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spelling doaj-art-eac7742f79164e458734d47a71cf2db12025-01-20T07:19:51ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602025-01-011210.3389/fped.2024.15057661505766Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literatureElio Paris0Giacomo De Marco1Oscar Vazquez2Sana Boudabbous3Christina Steiger4Romain Dayer5Dimitri Ceroni6Faculty of Medicine, University of Geneva, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, SwitzerlandRadiology Department, Geneva University Hospital, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, SwitzerlandPediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, SwitzerlandFibrous dysplasia (FD) is a benign medullary fibro-osseous anomaly that compromises the mechanical strength of bones, especially the long bones that bear strong mechanical stresses. It can lead to an inability to remodel immature bone into mature lamellar bone, resulting in inappropriate bone alignment in response to mechanical stresses. This case study describes a rare case of polyostotic FD presenting with an epiphyseal lesion of the proximal femoral head in its weight-bearing zone, accompanied by an unconventional femoral malrotation. The present case leads us to recommend that clinicians should not underestimate the occurrence of other deformities, such as the retrotorsion or flexion deformities that can compromise bone structure and the hip's biomechanics. Finally, the involvement of the epiphysis is probably more common than usually thought, introducing an additional complexity since juxta-articular lesions in weight-bearing joints may collapse, compromising articular congruence and function. To minimise this risk, bone scintigraphy and MRI should play a critical role in the patient's workup, evaluation, prognosis and follow-up.https://www.frontiersin.org/articles/10.3389/fped.2024.1505766/fullfibrous dysplasiaproximal femurpediatriccase reportepiphysis
spellingShingle Elio Paris
Giacomo De Marco
Oscar Vazquez
Sana Boudabbous
Christina Steiger
Romain Dayer
Dimitri Ceroni
Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literature
Frontiers in Pediatrics
fibrous dysplasia
proximal femur
pediatric
case report
epiphysis
title Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literature
title_full Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literature
title_fullStr Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literature
title_full_unstemmed Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literature
title_short Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literature
title_sort polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child a case report and review of the literature
topic fibrous dysplasia
proximal femur
pediatric
case report
epiphysis
url https://www.frontiersin.org/articles/10.3389/fped.2024.1505766/full
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